Sjögren’s syndrome

Definition/diagnostic criteria Sjögren’s syndrome is a chronic, multi-system autoimmune rheumatic disease predominantly affecting moisture-producing glands. It causes dryness of the mouth and eyes, and can lead to systemic effects impacting various organs. It is often underdiagnosed due to non-specific presenting complaints​​.

Epidemiology This condition affects approximately 0.3–0.5% of the adult population in the western world, making it the second most common autoimmune rheumatic disease. Women are nine times more likely to be affected than men. The actual prevalence may be higher, as many cases remain undiagnosed​​.

Diagnosis
Clinical features: The diagnosis of Sjögren’s Syndrome can be challenging due to the overlap of its symptoms with other conditions. Common symptoms include dry eyes and mouth, fatigue, and musculoskeletal pain. GPs should be alert to these symptoms and consider a specialist referral for further evaluation​​.

Investigations: Key investigations include:

• Blood tests: anti-ro/SSA and anti-la/SSB antibodies are commonly positive. It is worth checking with your local pathology department as the tests performed may vary, and the spectrum of autoantibodies is continually evolving.
• Eye examination: An ophthalmologist may conduct a test to examine the tear layer on the eyes, looking for signs indicative of the syndrome​​.
• Other tests: Include measuring saliva and tear production​​ and salivary gland biopsy.

Treatment

• Patients should be referred to secondary care.
• Dry eyes and mouth management: Focuses on replacing reduced secretions.
• Avoid medication that worsens symptoms (e.g anticholinergics).
• Systemic disease treatment as required (e.g. nifedipine for associated Raynaud’s syndrome or immunosuppressants for associated vasculitis).

Prognosis Sjögren’s syndrome is a long-term condition that can vary in its impact on patients. While some may experience minor inconvenience, others may face significant life disruptions. Complications can include vision and lung problems, and there is a slightly increased risk of non-Hodgkin lymphoma​​.